Table 2.

Human ADAMTS Family Members, Gene Locus, Molecular Weight, Tissue Distribution, and Substrates.

ADAMTS	Gene Locus	MW (kDa)	Tissue Distribution	Substrate/Target	Biological or Pathological Role	Ref
1	21q21	105	Heart, bronchial epithelial cells, fetal lung, liver, aorta, smooth muscle, colon, uterus, kidney, adrenal gland, adipocytes, placenta, uterus, ovary, prostate, bladder, spinal cord, ciliary ganglion, olfactory bulb, breast stromal fibroblasts and myoepithelial cells	Aggrecan, versican, syndecan 4, TFPI-2, semaphorin 3C, nidogen-1, −2, desmocollin-3, dystroglycan, mac-2, gelatin (denatured collagen type I), amphiregulin, TGF-α, heparin-binding EGF, VEGF	Cancer (both pro- and antitumorigenic/metastatic), antiangiogenic	[172–174]
2	5q35	135	Breast stromal fibroblasts adipocytes, heart, lung, liver, kidney, bladder, aorta, smooth muscle, skeletal muscle, tendon, bone, skin, retina, superior cervical ganglion, uterus, placenta	Fibrillar procollagens types I, II, III and V N-propeptide	Ehlers-Danlos syndrome type VIIc, dermatosparaxis (in sheep and cattle)	[41, 172, 175]
3	4q21	140–150	CD105+ endothelial cells, CD34+ cells, heart, lung, pineal gland, cartilage, bone, skeletal muscle, tendon, breast myoepithelial cells, placenta, brain	Fibrillar procollagen type II N-propeptide, biglycan, pro-VEGF-C, reelin	Lymphangiogenesis, placental angiogenesis, brain functions	[11, 41, 107, 129, 172, 176, 177]
4	1q23	90	Ovary, adrenal cortex, ciliary ganglion, trigeminal ganglion, brain, spinal cord, retina, pancreas (islets), heart, fetal lung, bladder, uterus, skeletal muscle, breast myoepithelial cells, synovial fluid	Aggrecan, versican, neurocan, reelin, biglycan, brevican, matrilin-3, α2-macroglobulin, oligomeric matrix protein (COMP)	Arthritis	[44, 172, 178]
5 (11)	21q21	73	Adipocytes, bladder, uterus, placenta, breast myoepithelial cells	Aggrecan, versican, reelin, biglycan, matrilin-4, brevican, α2-macroglobulin	Osteoarthritis, cancer (anti-tumorigenic, anti-angiogenic)	[44, 172, 179, 180]
6	5q12	116	Superior cervical ganglion, trigeminal ganglion, appendix, heart, breast myoepithelial cells	TBD	TBD	[172, 180]
7	15q24	182	Trigeminal ganglion, adrenal cortex, kidney, liver, pancreas, heart, smooth muscle, skeletal muscle, intervertebral disc, breast stromal fibroblasts	Cartilage oligomeric matrix protein (COMP)	Coronary artery disease (smooth muscle cell migration)	[172, 180, 181]
8	11q25	80	Skeletal muscle, heart, lung, liver, superior cervical ganglion, adrenal cortex, breast stromal fibroblasts and luminal epithelial cells, placenta, brain	Aggrecan	Anti-angiogenic	[172, 174]
9	3p14	217	Heart, lung, kidney, pancreas, colon, ovary, skeletal muscle, dorsal root ganglion, capillary endothelial cells, breast myoepithelial cells	Aggrecan, versican	Cancer	[172, 182–184]
10	19p13	121	Heart, lung, liver, pancreas, kidney, brain, placenta, CD8+ T-cells, brain, uterus, breast stromal fibroblasts	Fibrillin-1	Weill-Marchesani syndrome	[172, 185, 186]
12	5q35	178	Liver, bone marrow, intervertebral disc, atrioventricular node, smooth muscle, breast stromal fibroblasts and myoepithelial cells	oligomeric matrix protein (COMP)	Cell adhesion, cancer	[172, 187]
13	9q34	190	Heart, lung, liver, pancreas. kidney, brain, placenta, CD71+ early erythroid cells, endothelial cells, thyroid, breast myoepithelial cells	von Willebrand factor	Deficiency leads to thrombotic thrombocytopenic purpura	[43, 110, 172, 188–191]
14	10q21	134	Fibroblasts, lung, liver, prostate, retina, placenta, thalamus, bone marrow, fetal thyroid, adipocytes, cerebellum, bone, skin, breast myoepithelial and luminal epithelial cells	Fibrillar procollagen type I N-propeptide (pNα1 and pNα2 chains)	Linked to multiple sclerosis, osteoarthritis in women	[41, 112–114, 172, 190]
15	11q25	103	Colon, brain, heart, uterus, musculoskeletal system, breast myoepithelial cells	Aggrecan, versican	Cancer (anti-tumorigenic/metastatic, anti-angiogenic)	[172, 192]
16	5p15	136	Brain, ovary, breast myoepithelial cells, aorta	TBD	Hypertension	[172, 190]
17	15q24	121	Ovary, breast myoepithelial cells	TBD	Weill-Marchesani-like syndrome	[172, 190]
18	16q23	135	Endothelium, prostate, brain, ciliary ganglion, heart, skin, breast myoepithelial cells	TBD	Microcornea, myopic chorioretinal atrophy and telecanthus	[43, 44, 142, 172, 190]
19	5q31	134	Dorsal root ganglion, breast myoepithelial cells	TBD	TBD	[172]
20	12q12	215	Heart, lung, pancreas, prostate, testis, ovary, placenta, brain, appendix, liver, skeletal muscle, pituitary, trigeminal ganglion, breast myoepithelial cells	Versican	Colorectal cancer	[172, 183, 193]

MW, molecular weight of full-length protein; TBD, to be determined